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Synovial Chondromatosis and Soft Tissue Chondroma: Extraosseous Cartilaginous Tumor Defined by FN1 Gene Rearrangement

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Synovial Chondromatosis and Soft Tissue Chondroma: Extraosseous Cartilaginous Tumor Defined by FN1 Gene Rearrangement Modern Pathology (2019) 32:1762–1771 https://doi.org/10.1038/s41379-019-0315-8 ARTICLE Synovial chondromatosis and soft tissue chondroma: extraosseous cartilaginous tumor defined by FN1 gene rearrangement 1 1 1 2 1 3 Fernanda Amary ● Luis Perez-Casanova ● Hongtao Ye ● Lucia Cottone ● Anna-Christina Strobl ● Paul Cool ● 2 1 1 1,2 1 1,2 Elena Miranda ● Fitim Berisha ● William Aston ● Maia Rocha ● Paul O’Donnell ● Nischalan Pillay ● 1 4 2 1,2 Roberto Tirabosco ● Daniel Baumhoer ● Edward S. Hookway ● Adrienne M. Flanagan Received: 31 March 2019 / Revised: 4 June 2019 / Accepted: 4 June 2019 / Published online: 4 July 2019 © The Author(s), under exclusive licence to United States & Canadian Academy of Pathology 2019 Abstract A fusion between fibronectin 1 (FN1) and activin receptor 2A (ACVR2A) has been reported previously in isolated cases of the synovial chondromatosis. To analyze further and validate the findings, we performed FISH and demonstrated recurrent FN1–ACVR2A rearrangements in synovial chondromatosis (57%), and chondrosarcoma secondary to synovial chondromatosis (75%), showing that FN1 and/or AVCR2A gene rearrangements do not distinguish between benign and malignant synovial chondromatosis. RNA sequencing revealed the presence of the FN1–ACVR2A fusion in several cases 1234567890();,: 1234567890();,: that were negative by FISH suggesting that the true prevalence of this fusion is potentially higher than 57%. In soft tissue chondromas, FN1 alterations were detected by FISH in 50% of cases but no ACVR2A alterations were identified. RNA sequencing identified a fusion involving FN1 and fibroblast growth factor receptor 2 (FGFR2) in the case of soft tissue chondroma and FISH confirmed recurrent involvement of both FGFR1 and FGFR2. These fusions were present in a subset of soft tissue chondromas characterized by grungy calcification, a feature reminiscent of phosphaturic mesenchymal tumor. However, unlike the latter, fibroblast growth factor 23 (FGF23) mRNA expression was not elevated in soft tissue chondromas harboring the FN1–FGFR1 fusion. The mutual exclusivity of ACVR2A rearrangements observed in synovial chondromatosis and FGFR1/2 in soft tissue chondromas suggests these represent separate entities. There have been no reports of malignant soft tissue chondromas, therefore differentiating these lesions will potentially alter clinical management by allowing soft tissue chondromas to be managed more conservatively. Introduction Synovial chondromatosis is a rare benign neoplasm asso- ciated with the synovium of a joint, tendon sheath, or bursa. The presence of endochondral ossification in up to 50% of These authors contributed equally: Fernanda Amary, Luis Perez- cases led to the synonym synovial osteochondromatosis [1]. Casanova Any synovial joint can be involved, but it is more common These authors contributed equally: Edward S Hookway, Adrienne M in larger joints including the knee, hip, and elbow. The Flanagan lesion presents most commonly over 40 years of age and Supplementary information The online version of this article (https:// there is a male predominance (2:1). Histologically, synovial doi.org/10.1038/s41379-019-0315-8) contains supplementary chondromatosis is characterized by multiple hyaline material, which is available to authorized users. * Edward S. Hookway 2 Cancer Institute, 72 Huntley Street, University College London, [email protected] London WC1E 6BT, UK * Adrienne M. Flanagan 3 a.fl[email protected] The Robert Jones and Agnes Hunt Orthopaedic Hospital NHS Foundation Trust, Oswestry SY10 7AG, UK 4 1 Royal National Orthopaedic Hospital NHS Trust, Bone Tumor Reference Center, Institute of Pathology, University Stanmore, Middlesex HA7 4LP, UK Hospital Basel, Basel, Switzerland Synovial chondromatosis and soft tissue chondroma: extraosseous cartilaginous tumor defined by FN1. 1763 cartilage nodules intimately related to the synovium. These from the RJAH biobank were approved by the National may be embedded within the subsynovial tissue but may Research Ethics Committee of the Health Research also lie in the synovial fluid either attached to the synovium Committee (reference 17/YH/0108; IRAS project identi- or as ‘floating’ bodies. fier: 217446 and licensed by the HTA under number The recent findings of FN1–ACVR2A and ACVR2A–FN1 12073). Ethical approval was also given by the Ethik- in-frame fusions in a case of chondrosarcoma arising sec- kommission bei der Basel (reference 274/12). ondary to synovial chondromatosis and in one of seven All samples were diagnosed by specialist bone tumor cases of synovial chondromatosis [2], indicate that these pathologists (AMF, DB, FA, and RT) according to the lesions represent neoplasms, and that they rarely transform World Health Organization classification 2013 [13, 14]. into secondary chondrosarcoma [3–6]. However, the find- When available, cases were correlated with clinical and ings by Totoki et al., to the best of our knowledge, have not imaging data. All samples were fixed in 10% formal saline, yet been validated. Since peripheral and central chon- decalcified in EDTA or in nitric acid (5%), and processed in drosarcoma, chondroblastoma, phosphaturic mesenchymal paraffin: tissue sections were stained with haematoxylin tumor, and extraskeletal mesenchymal chondrosarcoma are and eosin. among the potential differential diagnoses of synovium- related cartilage tumors, a reliable biomarker could aid Fluorescence in situ hybridization (FISH) significantly in the diagnostic accuracy of these lesions, particularly in needle core biopsies [7–10]. FISH was carried out as described previously [16]. In brief, Soft tissue chondromas occur most commonly in the sections were marked by a specialist histopathologist to hands and feet and share histological features with synovial target tumor-rich area, then deparaffinized and pretreated by chondromatosis, hence it is one of the main differential pressure-cooking following incubation in pepsin solution at diagnoses at these sites. Such lesions are considered to 37 °C for 50 min. Probes were added to tissue sections, represent distinct entities [11–14] and soft tissue chon- denatured at 72 °C, and hybridized overnight at 37 °C. dromas are by definition not related to the synovial lining. Thereafter, the sections were washed and counterstained Nevertheless, even with the use of high-resolution radi- with 4′, 6-diamidino-2-phenylindole (DAPI) and mounted ological imaging in individual cases it can be practically with coverslips. impossible to exclude unequivocally an association with a Probes used for FISH included Agilent SureFISH tendon sheath, a bursa lining, or synovium in the hands and custom-designed FN1 and ACVR2A break-apart probes feet. A specific biomarker would therefore aid in rendering (Agilent, Cheshire, UK) (Table S1) and commercially the correct diagnosis [15]. available break-apart probes for FGF1 (CytoTest, Rock- In this study we set out to determine the prevalence of ville, USA), FGFR1 (Leica Biosystems, Wetzlar, Ger- FN1 and ACVR2A gene rearrangements in a larger set of many), fibroblast growth factor receptor 2 (FGFR2) synovial chondromatosis, and secondary synovial chon- (ZytoVision, Bremerhaven, Germany), and p16/CDKN2A drosarcoma, and to search for recurrent genetic alterations (9p21) (Vysis, Abbott Molecular, IL, USA). A positive in soft tissue chondromas. result was obtained when at least 15% of the nuclei ana- lyzed reveal an aberrant signal on counting a minimum of 50 consecutive nonoverlapping nuclei. Assessment of p16/ Materials and methods CDKN2A FISH was undertaken as previously reported [17]. Samples previously known to have rearrangement for Cases were identified by searching the histopathology FGFR1, FGF1, and FGFR2 by FISH and/or RNA files at the Royal National Orthopaedic Hospital (RNOH), sequencing were employed as controls. UK, The Robert Jones and Agnes Hunt Orthopaedic Hospital (RJAH), and the Basel Bone Tumor Reference RNAscope Centre, Switzerland. The RNOH Biobank was approved by the National Research Ethics Committee of the Health The expression of FGF23 was assessed using the RNA- Research Committee (reference 15/YH/0311: Integrated scope™ assay (Advanced Cell Diagnostics, Hayward, CA, Research Application System (IRAS) project identifier: USA) according to the manufacturer’s instructions. Sections 18309). This specific project was approved by the were hybridized with a probe specific for FGF23 (Hs- National Research Ethics Committee approved UCL/ FGF23, Cat. 557241), and for the endogenous control UCLH Biobank Ethics Committee (specificproject Ubiquitin C (Hs-UBC, Cat. 310041). Hybridization signals reference no. EC17.14). This biobank was licensed by the were amplified and visualized with the RNAscope® 2.5 HD Human Tissue Authority under number 12073. Cases Detection Kit (Brown; Cat. 322360). 1764 F. Amary et al. Quantitative real time PCR (qPCR) Microsystems, Milton Keynes, UK) using the rabbit monoclonal histone H3F3 K36M mutant antibody (clone RNA was extracted from FFPE sections using the Ambion RM193; RevMAb Biosciences USA, San Francisco, CA, RecoverAll™ Total Nucleic Acid Isolation kit (Ambion, Austin, USA). Leica epitope retrieval 2 solution was used for 20 TX, USA) and reverse transcribed into cDNA with the Invi- min. The antibody was diluted 1:400 [8]. trogen™ SuperScript™ IV First-Strand Synthesis System with random hexamers (Thermo Fisher Scientific, Loughborough, Leicestershire, UK) according to manufacturer’s instructions. Results qPCR was carried out using TaqMan® Gene Expression Assays (Thermo Fisher
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